AWilms’TumorwithSpinalCordCompression:An ExtrarenalOrigin?downloads.hindawi.com/journals/cripe/2018/1709271.pdf · poleoftheleftkidney,deviatingittowardsthemidline.e mass is located

Case ReportA Wilms’ Tumor with Spinal Cord Compression: AnExtrarenal Origin?

Audrey Petit,1 Amandine Rubio,1 Chantal Durand,2 Christian Piolat,3 Cecile Perret,1

Anne Pagnier,1 Dominique Plantaz,1 and Herve Sartelet 4

1Departement de Pediatrique, CHU de Grenoble, Grenoble, France2Departement de Radiologie, CHU de Grenoble, Grenoble, France3Departement de Chirurgie, CHU de Grenoble, Grenoble, France4Departement de Pathologie, CHU de Grenoble, Grenoble, France

Correspondence should be addressed to Herve Sartelet; [email protected]

Received 4 May 2018; Accepted 5 August 2018; Published 3 September 2018

Academic Editor: Denis A. Cozzi

Copyright © 2018 Audrey Petit et al. ,is is an open access article distributed under the Creative Commons Attribution License,which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Spinal cord compression inWilms’ tumor (WT) is an extremely rare event that can have a very poor prognosis if not taken care ofrapidly. Most cases reported in the literature involve widely metastatic patient with bone or paraspinal metastases or occasionallyintradural metastasis. Here, we present the case of a 3-year-old girl of WTconfirmed by biopsy, with spinal cord compression dueto the direct contiguous spread of a tumor through 2 vertebral foramina. Abdominal ultrasonography and magnetic resonanceimaging performed for an abdominal mass revealed a large heterogeneous tumor near the upper pole of the left kidney. A nodularinfiltration extended through the T11-L1 and L1-L2 neural foramina, forming an intraspinal mass that compressed the spinal cord.Major paresthesia subsequently occurred, requiring urgent treatment with corticosteroids and chemotherapy. ,e evolution wasrapidly satisfying. After six courses of chemotherapy, a left nephrectomy was performed. Macroscopic examination identifieda large tumor attached to the kidney without renal infiltration. Microscopical examination concluded to a nephroblastoma withregressive changes, of intermediate risk. Evolution at 6 months is satisfactory, with no neurological deficit. ,e histological aspectof the tumor and the clinical outcome suggest that she had an extrarenal WT that spread through the vertebral foramina and wassecondarily attached to the kidney.

1. Introduction

Childhood renal neoplasm accounts for approximately 7%of all cancers in childhood and are in the vast majorityWilms’ tumor (WT) or nephroblastoma [1, 2]. About 10% ofWTpresent with heamatogenous spread, most commonly tothe lungs (85%), liver (10%) and only very rarely to the bones(1%) and brain [1, 2]. ,e occurrence of spinal cord com-pression ranges from 2.7 to 4% in childhood neoplasm,generally in metastatic or invasive Ewing’s sarcoma, oste-ogenic sarcoma, rhabdomyosarcoma, neuroblastoma, andlymphoma [3, 4]. Spinal cord compression may result inpermanent neurological deficit, further aggravating theburden of disease.

In the course of WT, spinal cord compression is a veryrare occurrence, usually involving skeletal metastases to

the vertebral body, intradural or extradural metastases[5–11].

Here, we report the case of a large WT in a 3-year-oldpatient with secondary spinal compression by direct con-tiguous spread through 2 vertebral foramina.

2. Case Presentation

A 3-year-old girl, with no prior medical history, was admittedin our center with a three-week history of an abdominal massdiscovered by her mother. On physical examination, a firm,painless mass in the left flank was palpable. Complete ex-amination showed no other abnormality. In particular, noneurological deficit was detected.

Abdominal ultrasonography revealed a large heteroge-neous tumor of 69× 67× 97 cm originating from the upper

HindawiCase Reports in PediatricsVolume 2018, Article ID 1709271, 4 pageshttps://doi.org/10.1155/2018/1709271

pole of the left kidney, deviating it towards the midline. ,emass is located on the periphery of the upper pole ofthe kidney, and a vascular pedicle seemed to emerge fromthe renal sinus. No calcification or hemorrhagic compo-nent was found. Magnetic resonance imaging (MRI) andcomputed tomography (CT) showed an encapsulated tu-mor but with a nodular infiltration of the retroperitonealfatty tissues. It extended through the T11-T12 and T12-L1neural foramina, forming an intraspinal mass from T11to L1 and compressing the spinal cord (Figure 1). As-sessment of tumor extension revealed two infracentimetricmetastases in the lungs. ,e tumor and its extradural ex-tension showed a major hypermetabolic activity on posi-tron emission tomography (PET). Bone marrow aspirationuncovered no medullary involvement. ,e urine cat-echolamines, neural specific enolase, alpha-foetoprotein,and human chorionic gonadotropin were normal. Labo-ratory studies evidenced only a small rise in LDH(417 IU/L) and fibrinogen (7.2 g/L).

Considering this extremely unusual clinicoradiologicalpresentation, a posterior transcutaneous needle biopsy wasperformed, as recommended in the International Society ofPediatric Oncology renal study group SIOP-RTSG 2001protocol. ,e histopathologic features revealed a triphasicnephroblastoma, with no anaplastic feature.

Meanwhile, the patient started complaining of majorparesthesia and leg pain, requiring urgent treatment with

corticosteroids and chemotherapy. Due to the neurologicalthreat and the lung nodules, chemotherapy according to theSIOP-RTSG 2001 for stage IV nephroblastoma was ad-ministered, including three drugs (vincristine, actinomycineD, and doxorubicine).

,e patient’s evolution was rapidly satisfying, with therapid and complete receding of neurological symptoms. ,epreoperative assessment, after four courses of chemother-apy, indicated a massive regression of the tumor volumeby 53%, with measures of 67× 46× 77 cm, and a completedisappearance of the intraspinal extension.,e lung noduleswere no longer detected on CT imaging.

After six courses of chemotherapy, a left nephrectomywasperformed.Macroscopic examination identified a large tumorattached to the kidney, enclosed in a thick fibrous capsule.,emicroscopic examination concluded to a triphasic nephro-blastoma with regressive changes, of intermediate risk andwithout capsular rupture, thereby staging it as a stage I of theSIOP-RTSG 2001 classification.,e tumor consisted in tumorepithelial component (abortive tubules and glomeruli) sur-rounded by metanephric blastema and tumor immaturespindled cell stromawithout any anaplasia or emboli of tumorcells. ,e histology of the kidney was unremarkable withoutany nephrogenic rest. Postoperative treatment included 29weeks of chemotherapy with the same three drugs. After 24months of evolution, the child is in good health and has noneurological deficit.

(a) (b) (c)

(d) (e)

Figure 1: (a) Magnetic resonance imaging (MRI) showed an encapsulated tumor, with signs of capsular effraction at the upper pole of theleft kidney. (b)Magnetic resonance imaging (MRI) showed a tumor, forming an intraspinal mass from T11 to L1 and compressing the spinalcord (arrow). (c) ,e left nephrectomy presented macroscopically a large tumor attached to the kidney without renal infiltration. (d) ,emicroscopical examination confirmed the separation between the tumor and the kidney (Hematein-Eosin-Safran, ×100). (e) Wilm’s tumor,with regressive changes, of intermediate risk with only small areas of blastema (Hematein-Eosin-Safran, ×400).

2 Case Reports in Pediatrics

3. Discussion

,e occurrence of spinal cord compression in childhoodneoplasm ranges from 2.7 to 4% and is most often seen in theterminal phase of a widely metastatic cancer [5–8] Althoughrare cases of intraspinal and vertebrae metastasis have beenreported in WT, intraspinal extension by direct contiguousspreading in a child devoid of spinal dysraphism has veryinfrequently been described. Here, we reported a case of“dumbbell” WT extending through 2 neural foramina andforming an intraspinal mass from T11 to L1 with spinal cordcompression in a 3-year-old child.

,e most common tumors causing spinal cord com-pression are neuroblastomas, soft-tissue sarcomas, fol-lowed by osteogenic and Ewing sarcomas, lymphomas, andvery rarely leukemia and WT [3, 4]. ,e precise patho-genesis of the spinal cord compression is variabledepending on the type of tumor. Neuroblastoma is par-ticularly prone to develop a spinal cord compression viadirect contiguous spread from paravertebral disease due toits sympathetic origin, with a classical dumbbell aspect aswas evidenced here [12]. ,e most frequently reportedsymptoms of spinal compression include back pain,weakness, sensory loss with gait disturbance, sphincter, andautonomic dysfunction [3]. Early signs of cord compres-sion must imperatively be recognized, as prompt diagnosisand treatment are mandatory to decrease the risk of ir-reversible loss of neurologic function [3, 4].

In the course of WT, spinal cord compression is in-variably explained by skeletal metastasis to the vertebralbody, intradural or extradural metastasis, although solitarymetastases to the spine have been described [5–11]. Mostcases involve patients with widely metastatic diseases witha very poor prognosis [8]. Surviving patients often retainfunctional neurological deficits [8, 11].

Only two cases of spinal cord compression by con-tiguous spread of a WT through the neural foramina hadpreviously been described [13, 14]. Both cases also oc-curred in extrarenal WT (ERWT), which is a very rareform of WT, estimated to account for less than 1% ofall cases of WT. ,e hypothesized pathogenesis of ERWTis the development of ectopic nephrogenic rests intoa nephroblastoma. Its prognosis is rather good withmostly a favorable histology and an 11% of local re-currence rate [15]. Cojean et al. reported the case of a 2-month-old boy who developed an abdominal ERWTextending through the intervertebral foramina, encroach-ing the spinal cord [13]. ,e other case, described byGovender et al., involved a child with preexistent occultspinal dysraphism, which facilitated the extension of theERWT to the spinal canal [14].

Our patient did not have any spinal dysraphism. Wehypothesize that she had an ERWT that spread through thevertebral foramina and was secondarily attached to thekidney. Indeed the macroscopic description of this tumor,with no renal capsular effraction, fits the description of anERWT, even though no nephrogenic rests were found. ,ebest outcome of this patient could be further evidence of theextrarenal origin of this neoplasm.

Spinal cord compression is a very rare occurrence inWT,but it can have dramatic functional and vital consequences ifnot taken care of appropriately. Treatment comprises ofimmediate chemotherapy and corticosteroids to reduce thetumor size, followed by nephrectomy.

While the vast majority of WT cases with spinal cordcompression are explained by metastasis to the spinal canal(bone, extradural or intradural metastasis), we report thefirst case of contiguous spreading from the primary tumorthrough the neural foramina in a child devoid of spinaldysraphism. ,is case could be explained by the extrarenalorigin of the nephroblastoma.

Conflicts of Interest

,e authors declare that they have no conflicts of interest.

References

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[13] N. Cojean, N. Entz-Werle, D. Eyer et al., “Dumbbell neph-roblastoma: an uncommon cause of spinal cord compression,”Pediatric Archives, vol. 10, no. 12, pp. 1075–1078, 2003.

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